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Year : 2019  |  Volume : 46  |  Issue : 3  |  Page : 99-102

A case of relapsing and remitting ptosis

Department of Neurology, Care Hospitals, Nampally, Hyderabad, Telangana, India

Date of Web Publication28-Jan-2020

Correspondence Address:
Dr. Radhakrishna Hari
8-3-224, C1-Vora Towers, C89-Madhuranagar, Yousufguda Hyderabad – 500 038, Telangana
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jss.JSS_34_19

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Hypertrophic pachymeningitis is a chronic inflammation of the duramater. Idiopathic intracranial pachymeningitis is a rare disease, diagnosed after exclusion of all other possible etiologies like infections, non-infectious inflammatory diseases, malignancy and others. It needs confirmation by meningeal biopsy as the treatment includes prolonged course of immunosuppressant drugs. One such patient is described in this article where the diagnosis eluded for nearly ten years and the patient had to approach different specialists. Ultimately histopathological conformation by meningeal biopsy resulted in identification and treatment.

Keywords: Hypertrophic pachymeningitis, Immunosuppressant drugs, IgG4 related disease

How to cite this article:
Hari R, Padhy BP, Reddy HM. A case of relapsing and remitting ptosis. J Sci Soc 2019;46:99-102

How to cite this URL:
Hari R, Padhy BP, Reddy HM. A case of relapsing and remitting ptosis. J Sci Soc [serial online] 2019 [cited 2021 Jul 28];46:99-102. Available from: https://www.jscisociety.com/text.asp?2019/46/3/99/276998

  Introduction Top

Pachymeningitis is a chronic inflammation of the intracranial dural covering of the brain. There can be several causes, such as syphilis, tuberculosis, rheumatoid arthritis, Sjogren's syndrome, temporal arteritis, granulomatosis with vasculitis (Wegener's granuloma), sarcoidosis, mixed connective tissue disease, malignancy, renal failure,[1] and lastly an idiopathic variety of the disease where the cause is unknown. Idiopathic intracranial hypertrophic pachymeningitis (IIHPM) is a diagnosis of exclusion wherein all the other causes are excluded by various appropriate investigations. It also needs confirmation by histopathology of the meningeal biopsy specimen. Biopsy from an accessible site with computed tomography or magnetic resonance imaging (MRI)-documented enhancing and thickened dura mater is more likely to yield a positive etiological diagnosis. The dura is diffusely but asymmetrically involved and hence the site for biopsy can be selected from a contrast-enhanced scan. The irregularly thickened dura may sometimes mimic dural masses, such as en plaque meningioma. Although white matter is generally spared, sinus thrombosis and resultant venous congestion and white matter changes can occur occasionally.[2] Brain parenchyma is generally not involved, but small areas of the brain, when biopsied, may show necrosis and gliosis following venous congestion. Probably, Charcot was the first person to describe a case of idiopathic hypertrophic pachymeningitis in 1869.

Recurrent symptoms of nonspecific headache and vomiting are described in the follow-up IIHPM cases. Rarely, visual loss (due to constriction of optic nerves as they pass through optic canals) or hearing impairment is also seen on long-term follow-up.[2] There can sometimes be long periods of clinical silence in the protracted clinical course of IIHPM.

Two patterns of dural involvement are described – one involving the cavernous sinus and related cranial nerves II, III, IV, V, and VI and the other pattern involving falcotentorial dura and affecting the V, VII, VIII, IX, and X cranial nerves. The condition generally responds to corticosteroids with or without other steroid-sparing immunomodulatory drugs such as azathioprine. Some cases are described which are resistant to corticosteroid therapy and may require surgical decompression.

  Case Report Top

A 40-year-old female presented with episodes of diplopia spanning over the past 10 years. The diplopia was associated with ptosis in the right eye more than in the left eye. She also had progressive difficulty in swallowing for liquids more than for solids for the past 6 months and severe headache for the past 1 month before she attended neurology outpatient services of our hospital in December 2017.

Ten years back, the horizontal diplopia and ptosis were examined by an ophthalmologist who treated with oral prednisolone for about 1 month and she improved well. There were recurrences once in 1–2 years, and she used to visit the same ophthalmologist for treatment. There were a total of six episodes in the past 10 years. However, in November 2016, the diplopia did not resolve completely with oral steroids even after 2 months of meticulous use, and she was referred to a neurophysician in the early 2017. Two MRI brain scans were obtained in January and May 2017, and both were normal. She developed an additional symptom in the form of dysphagia, and she was referred to a higher center for evaluation. At the referral center, repetitive nerve stimulation of the right facial nerve and spinal accessory nerve (trapezius muscle) did not show any decremental response, but acetylcholine receptor antibodies (AChR-Ab) were positive at 1.601 nmol/L (negative <0.40 nmol/L). She was started on pyridostigmine 60 mg three times daily, oral prednisolone 60 mg daily, and azathioprine in addition to other supportive medicines in July 2017. There was partial symptomatic relief in the subsequent 2 months. The steroids and azathioprine were slowly tapered and stopped, and pyridostigmine was continued. However, her diplopia and bulbar symptoms worsened. A repeat AChR-Ab test was negative in October 2017 (<0.01 nmol/L).

Along with dysphagia and diplopia, she also developed holocranial, continuous headache with right-sided predominance and vomiting in November 2017. There was a temporary relief with nonsteroidal anti-inflammatory drugs. She attended our hospital 1 month after the onset of these new symptoms. On examination in the neurology outpatient department in December 2017, she was conscious and fully oriented with normal visual acuity and visual fields. There was bilateral papilledema. There was bilateral lateral rectus palsy; right superior rectus, medial rectus, and inferior oblique and superior oblique muscles also had restricted movement. There were no long tract signs and no meningeal or cerebellar signs. On December 15, a contrast-enhanced MRI brain scan was done, which showed smooth pachymeningeal enhancement overlying the right temporal convexity and tentorium cerebelli predominantly on the right side [Figure 1] and [Figure 2]. The cerebrospinal fluid was under pressure, and analysis showed 11 cells per/high-power field with 90% lymphocytes, protein of 93 mg/dl, and glucose of 72 mg/dl. A detailed workup for vasculitis, sarcoid, and other systemic diseases was negative. X-ray chest and abdominal ultrasound scan were negative for any significant pathology. Meningeal biopsy along with the adjacent brain parenchyma was obtained through a cranial burr hole from the right temporal region. The histopathological examination of the brain parenchyma was unremarkable, and meninges showed thickened dura and mild lymphocytic reaction [Figure 3], [Figure 4], [Figure 5], [Figure 6]. There were no malignancy and no transmural vasculitic reaction. Immunoglobulin (Ig) IgG4 immunostaining was also negative in the biopsy specimen. Serum IgG4 was normal. A diagnosis of IIHPM was considered.
Figure 1: Pachymeningeal enhancement

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Figure 2: Smooth pachymeningeal enhancement

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Figure 3: Thickened dura with focal inflammation (×10)

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Figure 4: Thick-walled vessel with lymphocytic infiltrate around vessel but no transmural inflammation (×40)

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Figure 5: Fibrosis (×10)

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Figure 6: Sparse lymphocytic infiltrate, no plasma cell predominance

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She was started on oral prednisolone 50 mg/day, acetazolamide, levetiracetam, and supportive medical regimen. The headache, bulbar symptoms, and diplopia resolved by 80% in 1 month and completely after 2 months. The papilledema too resolved. The steroids were tapered slowly, and azathioprine was introduced as a steroid-sparing agent. Nearly 1 year later, as the oral prednisolone was tapering at 15 mg/day, there was recurrence of diplopia [Figure 7] which recovered as the drug was escalated. She developed diabetes during this 1-year course, which is well controlled with metformin. She is under regular follow-up since then.
Figure 7: Right lateral rectus palsy during relapse

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  Discussion Top

Hypertrophic pachymeningitis is a rare disease of diverse etiology. Being a diffuse inflammatory disease involving the dura mater, it causes thickening of the dura and may cause symptoms by the way of inflammation, thickening, and constriction of the nearby structures. It can affect the cranial or spinal dura or both. In general, the symptoms are headache, cranial neuropathies, or ataxia, either alone or in various combinations.

IIHPM is best evaluated by MRI scan which can pick up the localized or diffusely thickened dura. Gadolinium contrast produces uniform enhancement of the thickened dura. The pathological findings include thick fibrous dura associated with chronic inflammatory cell infiltrates, comprising lymphocytes and plasma cells. Giant cells, caseation necrosis or epithelioid granuloma, or evidence of transmural inflammation suggestive of central nervous system vasculitis are not usually seen.

Headache is a common symptom in IIHPM. Although it appeared very late in the clinical history in our case, chronic daily headache is a presenting feature in 60% of the patients.[3] Failure to evaluate with gadolinium-enhanced MRI scan can result in diagnostic delay.

Extracranial mass lesions are uncommon in IIHPM. One case report of a nasal mass is available in the literature histologically proved to be hypertrophic pachymeningitis.[4] IgG4-related disease (IgG4 RD) is probably one of the common causes of IIHPM. In one series, it accounted for nearly 29% of cases.[5] In our case, the test was negative on immunostaining for IgG4 in the biopsy specimen. The clinical features and prognosis are the same for IgG4 RD and the idiopathic variety of the disease. Besides, IgG4 RD can produce lesions at multiple sites, either simultaneously or sequentially – in the brain, lungs, or kidneys. If not responding to the conventional corticosteroid therapy, the CD20 suppressor rituximab was found to be useful in the treatment of IgG4 RD.

IIHPM is a lifelong disease. It is responsive to corticosteroids. Although response to steroids is good in the initial attacks, the response wanes and may require other immunosuppressive drugs such as azathioprine in addition to steroids, in the later part of the disease.[6]

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Feringa ER, Weatherbee L. Hypertrophic granulomatous cranial pachymeningitis causing progressive blindness in a chronic dialysis patient. J Neurol Neurosurg Psychiatry 1975;38:1170-6.  Back to cited text no. 1
Lee YC, Chueng YC, Hsu SW, Lui CC. Idiopathic hypertrophic cranial pachymeningitis: Case report with 7 years of imaging follow-up. AJNR Am J Neuroradiol 2003;24:119-23.  Back to cited text no. 2
Wang YJ, Fuh JL, Lirng JF, Lu SR, Wang SJ. Headache profile in patients with idiopathic hypertrophic cranial pachymeningitis. Headache 2004;44:916-23.  Back to cited text no. 3
Salgotra B, Kishore K, Nirhale D, Sharma G. Idiopathic hypertrophic pachymeningitis presenting with a nasal mass: A rare case. Med J DY Patil University 2015;8:531-3.  Back to cited text no. 4
Wallace ZS, Carruthers MN, Khosroshahi A, Carruthers R, Shinagare S, Stemmer-Rachamimov A, et al. IgG4-related disease and hypertrophic pachymeningitis. Medicine (Baltimore) 2013;92:206-16.  Back to cited text no. 5
Kupersmith MJ, Martin V, Heller G, Shah A, Mitnick HJ. Idiopathic hypertrophic pachymeningitis. Neurology 2004;62:686-94.  Back to cited text no. 6


  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7]


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